1. Sickle Cell Crises
Steven Elsbecker DO
Peds ICU Presentation
November 15th 2013

2. What We Will Discuss
• Prevalence and epidemiology of Sickle Cell Disease
• Various Sickle Cell Disease complications
• The Sickle Cell Crises
• The diagnosis, management and disposition of Sickle Cell Crises

3. What We Won’t Discuss
• Chronic outpatient management
• Biochemical and molecular details
• Genetic inheritance details

4. Epidemiology
• Hgb SS affects 1:5000 Sub-Saharan African Americans
• 1:36000 Hispanic-Americans
• 44 States currently perform neonatal screening for sickle cell trait and disease
• 1:12 African Americans are carries of the sickle cell trait

5. Sickle Cell
Complications

6. Skin
• Stasis Ulcers

7. Neuro
• CVA 2/2 vaso-occlusion

8. Eye
• Retinopathy 2/2 vaso-occlusion

9. Pulmonary
• Embolism, Infarct, Infection

10. Vascular
• Vaso-occlusion causing downstream
ischemia anywhere in the body

11. Liver
• Hepatitis 2/2 blood transfusion
cholestasis, hepatic sequestration

12. Gall Bladder
• Cholelithiasis/Cholecystitis

13. Spleen
• Splenic Sequestration

14. Genitourinary
• Hematuria, decreased fertility,
impotence, priapism

15. Bone
• Infarct / Aseptic Necrosis
osteomyelitis, Aplastic Crisis

16. Reproductive
• Decreased fertility,
placental insufficiency

17. Immunologic
• Relative immunodeficiency

18. Erythrocytes
• Hemolysis

19. The Sickle Cell
Crises

20. The Crises
• These 4 general categories cause the most morbidity and mortality
• Splenic Sequestration Crisis
• Hemolytic Crisis
• Aplastic Crisis
• Vaso-occlusive Crisis

21. Splenic Sequestration Crisis
• Splenic engorgement
• Rapid drop in Hgb
• Circulatory collapse
• 3-24 hours
• Transfusion/Exchange

22. Hemolytic Crisis
• Acute, accelerated breakdown
• Oxidative stress
• Infection
• Hypoxia…
• More common with
concomitant G6PD deficiency

23. Aplastic Crisis
• Supply:Demand mismatch
• Most commonly follows Parvo B19
• Reticulocyte count
• Critical drops in Hgb

24. Vaso-Occlusive Crisis
• The cause of nearly all SCD complications
• Abnormal cells stack in small vessels
• Shunt = Ischemia = Pain
• Can cause any symptom 2/2
end organ ischemia

25. Acute Chest Syndrome
• Concomitant infarction and infection
• High morbidity and mortality
• Treat pain, hypoxia and infection
• Macrolides or Quinilones

26. Questions?
• Platt OS, Brambilla DJ, Rosse WF, et al. (June 1994). "Mortality in sickle cell disease. Life expectancy and risk factors for early death". N. Engl. J. Med. 330 (23): 1639–
44.doi:10.1056/NEJM199406093302303. ISSN 0028-4793. PMID 7993409.
• http://ericksontribune.com/2007/10/mds-launch-network-to-treat-sickle-cell/
• Wellems TE, Hayton K, Fairhurst RM (September 2009). "The impact of malaria parasitism: from corpuscles to communities". J. Clin. Invest. 119 (9): 2496–
505.doi:10.1172/JCI38307. PMC 2735907. PMID 19729847.
• Malowany JI, Butany J (February 2012). "Pathology of sickle cell disease". Seminars in Diagnostic Pathology 29 (1): 49–55. doi:10.1053/j.semdp.2011.07.005. PMID 22372205.
• "BestBets: How long should an average sickle cell crisis last?". Retrieved 2010-11-27.
• Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009-05-28). Robbins and Cotran Pathologic Basis of Disease, Professional Edition: Expert Consult - Online (Robbins
Pathology) (Kindle Locations 33498-33499). Elsevier Health. Kindle Edition.
• Glassberg J (August 2011). "Evidence-based management of sickle cell disease in the emergency department". Emergency Medicine Practice 13 (8): 1–20; quiz 20.PMID 22164362.
• Anie KA, Green J (2012). "Psychological therapies for sickle cell disease and pain". In Anie, Kofi A. Cochrane Database of Systematic Reviews (Online) 2:
CD001916.doi:10.1002/14651858.CD001916.pub2. PMID 22336781.